Glycerophosphoethanolamine concentration is elevated in brain of patients with dominantly inherited olivopontocerebellar atrophy.
We measured the concentration of glycerophosphoethanolamine (GPEA), a membrane breakdown product, in autopsied brain of 10 patients with dominantly inherited olivopontocerebellar atrophy (OPCA), a cerebellar ataxia disorder. As compared with the controls, mean GPEA levels were significantly elevated by 37-69% in 11 of the 15 brain areas examined, including extracerebellar brain regions in which no neuronal cell loss could be detected by semiquantitative estimation. Our data suggest the possibility of altered membrane phospholipid metabolism in OPCA which could be a contributing factor in the neuronal cell death.
Kish
S J
SJ
Clarke Institute of Psychiatry, Toronto, Ont., Canada.
Robitaille
Y
Y
Ball
M
M
Gilbert
J
J
Deck
J H
JH
Chang
L J
LJ
Schut
L
L
D000328:Adult; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D006801:Humans; D009849:Olivopontocerebellar Atrophies / Q000378:metabolism / Q000473:pathology*; D009928:Organ Specificity; D010714:Phosphatidylethanolamines / Q000032:analysis*; D012016:Reference Values
D010714:Phosphatidylethanolamines; C002449:glycerophosphoethanolamine